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GM00378 female affected Cystinosin sample

Identifiers
BioSample: SAMN00795020; Coriell: GM00378
Organism
Homo sapiens (human)
cellular organisms; Eukaryota; Opisthokonta; Metazoa; Eumetazoa; Bilateria; Deuterostomia; Chordata; Craniata; Vertebrata; Gnathostomata; Teleostomi; Euteleostomi; Sarcopterygii; Dipnotetrapodomorpha; Tetrapoda; Amniota; Mammalia; Theria; Eutheria; Boreoeutheria; Euarchontoglires; Primates; Haplorrhini; Simiiformes; Catarrhini; Hominoidea; Hominidae; Homininae; Homo
Attributes
cell lineGM00378
sexFemale
cell typeFibroblast
family id6
family roleproband
raceCaucasian
age7 YR
GeneCTNS
affected_byCystinosin
MutationIVS11+2
MutationT>C
MutationTRP138TER
collectionNIGMS Human Genetic Cell Repository
Tags
CTNS
Cystinosis, late-onset juvenile or adolescent nephropathic type
Cystinosin
Description

Conjunctiva and cornea contain typical cystinotic crystals;elevated white cell 1/2 cystine concentration;normal renal function at age 10;but by age 13 developed renal glomerular insufficiency;similarly affected brother;donor subject is a compound heterozygote: one allele has a G>A transition at nucleotide 753 in exon 7 of the CTNS gene 753G>A resulting in a substitution of a termination codon for tryptophan at codon 138 Trp138Ter (W138X) and a second allele has a splice site mutation at the +2 position of exon 11 IVS11+2 T>C resulting in the skipping of exon 11.

Submission
Coriell; 2012-03-05
Accession:
SAMN00795020
ID:
795020

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